Młodzieńcze idiopatyczne zapalenie stawów – Rokowania, prognozy i postęp choroby

Młodzieńcze idiopatyczne zapalenie stawów
Rokowania, prognozy i postęp choroby

Młodzieńcze idiopatyczne zapalenie stawów (MIZS) to heterogenna grupa chorób o zróżnicowanym przebiegu i rokowaniu, które uległo poprawie w erze leków biologicznych. Dane z kohorty ReACCh-Out wskazują, że przy medianie wieku 16,9 lat 47% pacjentów osiąga remisję bez leków, 25% pozostaje w remisji przy leczeniu, a 27% ma aktywną chorobę. Około 51% pacjentów stosuje co najmniej jeden lek przeciwreumatyczny, w tym 22% leki biologiczne. Czynniki prognostyczne niekorzystnego przebiegu obejmują m.in. ciężkość zapalenia stawów, symetryczne zajęcie stawów, wczesne zajęcie stawów biodrowych/nadgarstkowych, obecność czynnika reumatoidalnego oraz przedłużającą się aktywność choroby. Różne podtypy MIZS wykazują odmienne czynniki ryzyka, np. w postaci układowej utrzymujące się objawy i trombocytoza po 6 miesiącach, a w postaci skąpostawowej symetria zajęcia stawów i podwyższone OB. Wczesny wiek zachorowania (~6 lat) i utrzymujące się zapalenie stawów w pierwszym roku są związane z przetrwałym przebiegiem choroby.

Wprowadzenie do rokowania w młodzieńczym idiopatycznym zapaleniu stawów
Długookresowe wyniki leczenia w erze leków biologicznych
Czynniki predykcyjne przebiegu i rokowania MIZS

Ogólne czynniki predykcyjne niekorzystnego rokowania
Specyficzne czynniki w zależności od podtypu MIZS
Czynniki predykcyjne przetrwałego przebiegu w postaci układowej MIZS

Modele predykcyjne w MIZS

Nordycki model predykcyjny
Walidacja modeli predykcyjnych

Przewidywanie osiągnięcia nieaktywnej klinicznie choroby i trwałej remisji
Nowe kierunki w przewidywaniu przebiegu MIZS
Indywidualne różnice w przebiegu MIZS
Wyzwania w ocenie rokowania MIZS
Wnioski dotyczące rokowania w MIZS

Kolejne rozdziały

Wprowadzenie do rokowania w młodzieńczym idiopatycznym zapaleniu stawów

Młodzieńcze idiopatyczne zapalenie stawów (MIZS) stanowi heterogenną grupę chorób, których przebieg i rokowanie mogą znacząco się różnić w zależności od podtypu choroby i indywidualnych czynników pacjenta. Współczesne dane wskazują na poprawę rokowania w erze leków biologicznych, jednak nadal znaczna część pacjentów wchodzi w dorosłość z aktywną chorobą.¹²

Wczesne przewidywanie długoterminowego przebiegu MIZS ma kluczowe znaczenie dla optymalizacji strategii terapeutycznych. Badania pokazują, że odpowiednio wczesna identyfikacja czynników ryzyka niekorzystnego przebiegu choroby może pomóc w dostosowaniu leczenia do indywidualnego ryzyka niepełnosprawności u dzieci z MIZS.³⁴

Długookresowe wyniki leczenia w erze leków biologicznych

Badania kohorty ReACCh-Out przeprowadzone w kanadyjskich ośrodkach wykazały, że przy ostatniej wizycie kontrolnej (mediana wieku 16,9 lat): 47% pacjentów znajdowało się w remisji bez leków, 25% w remisji podczas przyjmowania leków, a 27% miało aktywną chorobę. Około 51% pacjentów przyjmowało co najmniej jeden lek przeciwreumatyczny, w tym 22% stosowało leki biologiczne.⁵

W porównaniu z historycznymi kohortami, obserwuje się istotną poprawę rokowania w MIZS, w tym mniejszy odsetek trwałych uszkodzeń stawów (tylko 1% pacjentów wymagało wymiany stawów), bardziej korzystne rokowanie dla układowej postaci MIZS oraz mniejszy odsetek progresji do rozszerzonej postaci skąpostawowej.⁶ Jednak pomimo dostępności nowoczesnych terapii, co czwarty pacjent wchodzi w dorosłość z aktywną chorobą, a co drugi nadal wymaga leczenia przeciwreumatycznego.⁷

Czynniki predykcyjne przebiegu i rokowania MIZS

Ogólne czynniki predykcyjne niekorzystnego rokowania

Badania zidentyfikowały szereg czynników związanych z niekorzystnym rokowaniem w MIZS:⁸

Większa ciężkość/rozległość zapalenia stawów na początku choroby
Symetryczny charakter zajęcia stawów
Wczesne zajęcie stawów biodrowych/nadgarstkowych
Obecność czynnika reumatoidalnego
Przedłużająca się aktywna choroba

⁹

Specyficzne czynniki w zależności od podtypu MIZS

Różne podtypy MIZS charakteryzują się odmiennymi czynnikami predykcyjnymi:¹⁰

Postać układowa MIZS: Utrzymujące się objawy układowe i trombocytoza w 6 miesięcy po zachorowaniu są związane z gorszym rokowaniem. Jednak ogólnie postać układowa ma obecnie lepsze rokowanie niż w przeszłości – badania wskazują, że 70% pacjentów osiąga remisję bez leków, a tylko 9% ma aktywną chorobę po średnio 5,7 roku od rozpoznania.¹¹¹²
Postać skąpostawowa: Symetria zajęcia stawów i wyższy poziom OB na początku choroby są związane z cięższym przebiegiem.¹³
Postać wielostawowa: Pacjenci mają odmienny profil receptorów ko-hamujących (co-IRs), z podwyższonym poziomem CTLA-4, PD-1 i 4-1BB w osoczu w porównaniu z innymi podtypami MIZS, co może wpływać na przebieg choroby.¹⁴

Czynniki predykcyjne przetrwałego przebiegu w postaci układowej MIZS

Badania z międzynarodowej kohorty JIRcohort wykazały, że:¹⁵

Wcześniejszy wiek zachorowania (około 6 lat) zwiększa ryzyko przetrwałego przebiegu choroby
Utrzymujące się zapalenie stawów w pierwszym roku choroby jest istotnie częstsze w grupie z przetrwałym przebiegiem
Potrzeba stosowania syntetycznych DMARDs może również prognozować przetrwały przebieg choroby, odzwierciedlając cięższy fenotyp
Częstość leczenia biologicznymi i syntetycznymi DMARDs jest wyższa w grupie z przetrwałym przebiegiem

¹⁶

Modele predykcyjne w MIZS

Nordycki model predykcyjny

Opracowano modele statystyczne do prognozowania długoterminowych niekorzystnych wyników w MIZS, które mogą ułatwić wczesne decyzje dotyczące leczenia. Modele nordyckie pozwalają przewidywać:¹⁷¹⁸

Nieosiągnięcie remisji bez leków
Niepełnosprawność funkcjonalną
Uszkodzenia stawów

Modele te opierają się na zmiennych łatwo dostępnych przy rozpoznaniu i mają akceptowalną czułość i swoistość. Ich główną zaletą jest łatwość stosowania, choć wymagają one walidacji w innych kohortach pacjentów z MIZS i wśród różnych grup etnicznych.¹⁹

Walidacja modeli predykcyjnych

Walidacja narzędzi predykcyjnych w populacjach innych niż te, w których zostały one pierwotnie opracowane, jest niezbędna do zrozumienia ich zastosowania w różnych systemach opieki zdrowotnej. Badania walidacyjne nordyckiego modelu predykcyjnego w kanadyjskiej kohorcie ReACCh-Out wykazały, że:²⁰

Po dostosowaniu współczynników, nordycki model przewidywania nieosiągnięcia remisji miał podobną zdolność predykcyjną u kanadyjskich pacjentów z MIZS
Nie udało się potwierdzić ważności nordyckiego modelu do przewidywania niepełnosprawności funkcjonalnej u kanadyjskich pacjentów

²¹

Przewidywanie osiągnięcia nieaktywnej klinicznie choroby i trwałej remisji

Identyfikacja czynników predykcyjnych dla osiągnięcia nieaktywnej klinicznie choroby (CID – clinically inactive disease) i trwałej remisji pozwoliłaby na szybsze wdrażanie intensywnego leczenia u dzieci z mniejszym prawdopodobieństwem osiągnięcia tych stanów.²²

W badaniu obejmującym 832 dzieci z MIZS, w ciągu pierwszego roku 31% osiągnęło CID według wstępnych kryteriów Wallacego, a 44% według wskaźnika cJADAS10 (26% osiągnęło CID według obu definicji). W ciągu pięciu lat od rozpoznania 60% kiedykolwiek osiągnęło trwałą remisję według kryteriów Wallacego, a 66% według wskaźnika cJADAS10 (52% osiągnęło trwałą remisję według obu definicji).²³

Zidentyfikowano różne czynniki predykcyjne dla CID według cJADAS10 i kryteriów Wallacego, choć podobne czynniki predykcyjne dla trwałej remisji przy wykorzystaniu obu definicji. Dzieci z:²⁴

Słabą wydolnością funkcjonalną
Wielostawową postacią MIZS
Młodszym wiekiem przy pierwszej prezentacji

mogłyby być leczone bardziej agresywnymi strategiami terapeutycznymi niż obecnie stosowane, w celu lepszej kontroli choroby.²⁵

Nowe kierunki w przewidywaniu przebiegu MIZS

Trwają badania nad rolą receptorów ko-hamujących limfocytów T (co-IRs) w patogenezie MIZS i ich potencjalnym wykorzystaniem jako markerów predykcyjnych oraz celów terapeutycznych.²⁶

Wykazano, że:

Pacjenci z wielostawową postacią MIZS mają odmienny profil co-IRs z wyższym poziomem CTLA-4, PD-1 i 4-1BB w osoczu niż inne podtypy MIZS
LAG-3 jest ważną cząsteczką w patogenezie skąpostawowej postaci MIZS
Agoniści LAG-3 mogą stanowić nową opcję terapeutyczną dla pacjentów ze skąpostawową postacią MIZS w przyszłości

²⁷

Indywidualne różnice w przebiegu MIZS

MIZS wpływa na każde dziecko inaczej. U niektórych choroba dotyka jednego lub dwóch stawów i jest łatwa do opanowania. U innych MIZS może obejmować wiele stawów i powodować cięższe lub dłużej trwające objawy.²⁸

Wczesne wykrycie i leczenie umożliwia opanowanie zapalenia stawów, zapobieganie uszkodzeniom stawów i zapewnienie normalnego lub prawie normalnego funkcjonowania większości dzieci z MIZS.²⁹

MIZS może trwać miesiące lub lata. Niektóre dzieci osiągają pełną remisję, podczas gdy inne nadal mają aktywne objawy w wieku dorosłym. Nie ma pewnego sposobu przewidzenia, czy tak się stanie, ale odpowiednia opieka medyczna i skuteczne leczenie mogą pomóc w przygotowaniu pacjenta do przyszłości.³⁰

Wyzwania w ocenie rokowania MIZS

Pomimo rosnącej ilości danych na temat czynników prognostycznych w MIZS, przewidywanie długoterminowego wyniku w pierwszych kilku miesiącach pozostaje trudne. Aby lepiej określić czynniki prognostyczne w przyszłych analizach, należy dążyć do zwiększenia standaryzacji między badaniami.³¹

Ponadto istnieje potrzeba opracowania systemu oceny radiograficznej i zestawu kryteriów remisji specyficznych dla MIZS, co mogłoby znacząco poprawić możliwości prognozowania przebiegu choroby.³²

Wnioski dotyczące rokowania w MIZS

Dane z różnych badań kohortowych sugerują, że rokowanie w MIZS w erze leków biologicznych uległo poprawie, ale wciąż istnieje znaczny odsetek pacjentów z niekorzystnym przebiegiem choroby:³³³⁴

Około 25-27% pacjentów wchodzi w dorosłość z aktywną chorobą
Około 50-51% pacjentów nadal wymaga leczenia przeciwreumatycznego w okresie wejścia w dorosłość
Obserwuje się zmniejszenie trwałych uszkodzeń stawów (tylko około 1% wymaga wymiany stawów)
Istnieje lepsze rokowanie dla układowej postaci MIZS, z ok. 70% pacjentów osiągających remisję bez leków
Modele predykcyjne mogą pomóc w identyfikacji pacjentów zagrożonych niekorzystnym przebiegiem i dostosowaniu terapii, choć wymagają dalszej walidacji

³⁵³⁶

Wczesne rozpoznanie czynników prognostycznych ma kluczowe znaczenie dla podejmowania decyzji terapeutycznych i może pomóc w indywidualizacji leczenia, co potencjalnie przyczyni się do lepszej kontroli choroby i poprawy długoterminowych wyników u pacjentów z MIZS.³⁷³⁸

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Materiały źródłowe

#1 Long-term outcomes and disease course of children with juvenile idiopathic arthritis in the ReACCh-Out cohort: a two-centre experience
https://pmc.ncbi.nlm.nih.gov/articles/PMC7733713/
To assess long-term outcomes of children with JIA diagnosed in the biologic era. […] At the last follow-up visit at a median age of 16.9 years, 47% were in remission off medications, 25% in remission on medications and 27% had active disease; 51% were on at least one anti-rheumatic medication (22% on biologics). […] Relative to historical cohorts, this study suggests a reduction in JIA permanent damage, a more favourable prognosis for systemic JIA and a lower progression to oligoarthritis extended category. However, in an era of biologic therapy, one in four patients with JIA still enter adulthood with active disease and one in two still on treatment. […] Relative to historical cohorts, young adults who were diagnosed with JIA early in the biologic era at two Canadian centres in this study had higher rates of remission and lower rates of permanent joint damage (1% required joint replacements).
#2 Long-term outcomes and disease course of children with juvenile idiopathic arthritis in the ReACCh-Out cohort: a two-centre experience
https://pmc.ncbi.nlm.nih.gov/articles/PMC7733713/
Our study found patients with systemic JIA had a good long-term prognosis with 70% in remission off medications and only 9% with active disease at a median of 5.7 years after diagnosis. […] This long-term follow-up of an inception cohort diagnosed early in the biologic era suggests a reduction in permanent damage across JIA categories, a more favourable prognosis for systemic JIA and a lower progression to oligoarthritis extended category than in historical cohorts. However, one in four patients still had disease activity in early adulthood and one in two were receiving anti-rheumatic medications.
#3 Predicting unfavorable long-term outcome in juvenile idiopathic arthritis: results from the Nordic cohort study | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-018-1571-6
The aim was to develop prediction rules that may guide early treatment decisions based on baseline clinical predictors of long-term unfavorable outcome in juvenile idiopathic arthritis (JIA). […] The feasibility of making long-term predictions of JIA outcome based on early clinical assessment is demonstrated. The prediction models have acceptable precision and require only readily available baseline variables. Further testing in other cohorts is warranted. […] The primary goal in the treatment of children with JIA is to achieve remission off medication, and the main implication of the current study is that prediction models may be useful in guiding decisions about treatment. […] In the Nordic JIA cohort, we have developed and evaluated prediction models for long-term unfavorable outcome with acceptable sensitivity and specificity based on variables easily available at baseline, which may guide individually tailored treatment. Prediction of long-term unfavorable outcome early in the disease course may be useful in deciding when to start aggressive treatment in JIA.
#4 Early predictors of outcome in juvenile idiopathic arthritis – PubMed
https://pubmed.ncbi.nlm.nih.gov/14969057/
The definition and management of „early arthritis” in children differ from those in adults because juvenile idiopathic arthritis (JIA) is markedly different from adult rheumatoid arthritis. Since a significant proportion of patients with JIA develop articular damage and enter adult life with persistently active disease, it is important to predict early in the disease course the long-term outcome in order to tailor treatment to the risk of disability. […] In summary, greater severity/extension of arthritis at onset, symmetric disease, precocious hip/wrist involvement, the presence of rheumatoid factor, and prolonged active disease were the best predictors of a poor outcome. Specific correlates for systemic JIA were persistent systemic features and thrombocytosis at 6 months following presentation, whereas joint symmetry and a higher erythrocyte sedimentation rate at onset were associated with a more severe course in oligoarticular JIA. However, although data is accumulating on prognostic factors in JIA, prediction of long-term outcome in the first few months remains difficult. To better define prognostic factors in future analyses, a considerable effort should be made to increase standardization among studies. Furthermore, a radiographic scoring system and a set of remission criteria specific for JIA should be developed.
#5 Long-term outcomes and disease course of children with juvenile idiopathic arthritis in the ReACCh-Out cohort: a two-centre experience
https://pmc.ncbi.nlm.nih.gov/articles/PMC7733713/
To assess long-term outcomes of children with JIA diagnosed in the biologic era. […] At the last follow-up visit at a median age of 16.9 years, 47% were in remission off medications, 25% in remission on medications and 27% had active disease; 51% were on at least one anti-rheumatic medication (22% on biologics). […] Relative to historical cohorts, this study suggests a reduction in JIA permanent damage, a more favourable prognosis for systemic JIA and a lower progression to oligoarthritis extended category. However, in an era of biologic therapy, one in four patients with JIA still enter adulthood with active disease and one in two still on treatment. […] Relative to historical cohorts, young adults who were diagnosed with JIA early in the biologic era at two Canadian centres in this study had higher rates of remission and lower rates of permanent joint damage (1% required joint replacements).
#6 Long-term outcomes and disease course of children with juvenile idiopathic arthritis in the ReACCh-Out cohort: a two-centre experience
https://pmc.ncbi.nlm.nih.gov/articles/PMC7733713/
To assess long-term outcomes of children with JIA diagnosed in the biologic era. […] At the last follow-up visit at a median age of 16.9 years, 47% were in remission off medications, 25% in remission on medications and 27% had active disease; 51% were on at least one anti-rheumatic medication (22% on biologics). […] Relative to historical cohorts, this study suggests a reduction in JIA permanent damage, a more favourable prognosis for systemic JIA and a lower progression to oligoarthritis extended category. However, in an era of biologic therapy, one in four patients with JIA still enter adulthood with active disease and one in two still on treatment. […] Relative to historical cohorts, young adults who were diagnosed with JIA early in the biologic era at two Canadian centres in this study had higher rates of remission and lower rates of permanent joint damage (1% required joint replacements).
#7 Long-term outcomes and disease course of children with juvenile idiopathic arthritis in the ReACCh-Out cohort: a two-centre experience
https://pmc.ncbi.nlm.nih.gov/articles/PMC7733713/
Our study found patients with systemic JIA had a good long-term prognosis with 70% in remission off medications and only 9% with active disease at a median of 5.7 years after diagnosis. […] This long-term follow-up of an inception cohort diagnosed early in the biologic era suggests a reduction in permanent damage across JIA categories, a more favourable prognosis for systemic JIA and a lower progression to oligoarthritis extended category than in historical cohorts. However, one in four patients still had disease activity in early adulthood and one in two were receiving anti-rheumatic medications.
#8 Early predictors of outcome in juvenile idiopathic arthritis – PubMed
https://pubmed.ncbi.nlm.nih.gov/14969057/
The definition and management of „early arthritis” in children differ from those in adults because juvenile idiopathic arthritis (JIA) is markedly different from adult rheumatoid arthritis. Since a significant proportion of patients with JIA develop articular damage and enter adult life with persistently active disease, it is important to predict early in the disease course the long-term outcome in order to tailor treatment to the risk of disability. […] In summary, greater severity/extension of arthritis at onset, symmetric disease, precocious hip/wrist involvement, the presence of rheumatoid factor, and prolonged active disease were the best predictors of a poor outcome. Specific correlates for systemic JIA were persistent systemic features and thrombocytosis at 6 months following presentation, whereas joint symmetry and a higher erythrocyte sedimentation rate at onset were associated with a more severe course in oligoarticular JIA. However, although data is accumulating on prognostic factors in JIA, prediction of long-term outcome in the first few months remains difficult. To better define prognostic factors in future analyses, a considerable effort should be made to increase standardization among studies. Furthermore, a radiographic scoring system and a set of remission criteria specific for JIA should be developed.
#9 Early predictors of outcome in juvenile idiopathic arthritis – PubMed
https://pubmed.ncbi.nlm.nih.gov/14969057/
The definition and management of „early arthritis” in children differ from those in adults because juvenile idiopathic arthritis (JIA) is markedly different from adult rheumatoid arthritis. Since a significant proportion of patients with JIA develop articular damage and enter adult life with persistently active disease, it is important to predict early in the disease course the long-term outcome in order to tailor treatment to the risk of disability. […] In summary, greater severity/extension of arthritis at onset, symmetric disease, precocious hip/wrist involvement, the presence of rheumatoid factor, and prolonged active disease were the best predictors of a poor outcome. Specific correlates for systemic JIA were persistent systemic features and thrombocytosis at 6 months following presentation, whereas joint symmetry and a higher erythrocyte sedimentation rate at onset were associated with a more severe course in oligoarticular JIA. However, although data is accumulating on prognostic factors in JIA, prediction of long-term outcome in the first few months remains difficult. To better define prognostic factors in future analyses, a considerable effort should be made to increase standardization among studies. Furthermore, a radiographic scoring system and a set of remission criteria specific for JIA should be developed.
#10 Early predictors of outcome in juvenile idiopathic arthritis – PubMed
https://pubmed.ncbi.nlm.nih.gov/14969057/
The definition and management of „early arthritis” in children differ from those in adults because juvenile idiopathic arthritis (JIA) is markedly different from adult rheumatoid arthritis. Since a significant proportion of patients with JIA develop articular damage and enter adult life with persistently active disease, it is important to predict early in the disease course the long-term outcome in order to tailor treatment to the risk of disability. […] In summary, greater severity/extension of arthritis at onset, symmetric disease, precocious hip/wrist involvement, the presence of rheumatoid factor, and prolonged active disease were the best predictors of a poor outcome. Specific correlates for systemic JIA were persistent systemic features and thrombocytosis at 6 months following presentation, whereas joint symmetry and a higher erythrocyte sedimentation rate at onset were associated with a more severe course in oligoarticular JIA. However, although data is accumulating on prognostic factors in JIA, prediction of long-term outcome in the first few months remains difficult. To better define prognostic factors in future analyses, a considerable effort should be made to increase standardization among studies. Furthermore, a radiographic scoring system and a set of remission criteria specific for JIA should be developed.
#11 Long-term outcomes and disease course of children with juvenile idiopathic arthritis in the ReACCh-Out cohort: a two-centre experience
https://pmc.ncbi.nlm.nih.gov/articles/PMC7733713/
Our study found patients with systemic JIA had a good long-term prognosis with 70% in remission off medications and only 9% with active disease at a median of 5.7 years after diagnosis. […] This long-term follow-up of an inception cohort diagnosed early in the biologic era suggests a reduction in permanent damage across JIA categories, a more favourable prognosis for systemic JIA and a lower progression to oligoarthritis extended category than in historical cohorts. However, one in four patients still had disease activity in early adulthood and one in two were receiving anti-rheumatic medications.
#12 Disease evolution in systemic juvenile idiopathic arthritis: an international, observational cohort study through JIRcohort | Pediatric Rheumatology | Full Text
https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-023-00886-9
Systemic juvenile idiopathic arthritis (systemic JIA) is a severe disease with both systemic and joint inflammation. This study aims to identify predictors of disease evolution within the systemic JIA population enrolled in the Juvenile Inflammatory Rheumatism cohort (JIRcohort). […] Our results suggest that the earlier disease onset, the persistence of arthritis throughout the first year of disease evolution and the need of sDMARD might predict a persistent disease course. […] The patients of the persistent disease evolution group trended to have the disease onset at a younger age. […] Active arthritis was significantly more often seen in the persistent group at 12 months of diagnosis. […] The treatment frequency with bDMARD and sDMARD was higher in the persistent group. […] In our model parameters like young age at disease onset (6 years) and active arthritis at 12 months, were found to potentially have an influence on the risk of a persistent disease evolution during the first 24 months after diagnosis, even if the association was no longer statistically significant after adjusting for confounding factors. The need of sDMARD use may also predict a persistent disease course, reflecting a more severe phenotype.
#13 Early predictors of outcome in juvenile idiopathic arthritis – PubMed
https://pubmed.ncbi.nlm.nih.gov/14969057/
The definition and management of „early arthritis” in children differ from those in adults because juvenile idiopathic arthritis (JIA) is markedly different from adult rheumatoid arthritis. Since a significant proportion of patients with JIA develop articular damage and enter adult life with persistently active disease, it is important to predict early in the disease course the long-term outcome in order to tailor treatment to the risk of disability. […] In summary, greater severity/extension of arthritis at onset, symmetric disease, precocious hip/wrist involvement, the presence of rheumatoid factor, and prolonged active disease were the best predictors of a poor outcome. Specific correlates for systemic JIA were persistent systemic features and thrombocytosis at 6 months following presentation, whereas joint symmetry and a higher erythrocyte sedimentation rate at onset were associated with a more severe course in oligoarticular JIA. However, although data is accumulating on prognostic factors in JIA, prediction of long-term outcome in the first few months remains difficult. To better define prognostic factors in future analyses, a considerable effort should be made to increase standardization among studies. Furthermore, a radiographic scoring system and a set of remission criteria specific for JIA should be developed.
#14 FOREUM â Development of new tools for prediction and prevention of RA (PREDICT RA)
https://www.foreum.org/P017_pre_predictRA.cfm?projectid=153
Juvenile idiopathic arthritis (JIA) is a chronic inflammatory disease that can cause severe disability and even mortality with joint swelling, sensitivity, loss of motion and synovial tissue damage. […] We aim to define the role of T cell co-inhibitory receptors (co-IRs) for predicting the outcome of JIA and try to find a novel therapeutic target molecule. […] This is the first study showing the role of co-inhibitory receptors (checkpoint proteins) in the pathogenesis of JIA. […] Polyarticular JIA patients had a different coIR profile, having more CTLA-4, PD-1 and 4-1BB in their plasma than the other subtypes of JIA. […] We have shown that LAG-3 is an important molecule in the pathogenesis of oligoarticular JIA. […] We have shown that LAG3 agonists might be a novel therapeutic option for oligoarticular JIA patients in the future. […] This is the first study analysing these proteins in childhood arthritis and is hoped to lead to more work in the relevant area.
#15 Disease evolution in systemic juvenile idiopathic arthritis: an international, observational cohort study through JIRcohort | Pediatric Rheumatology | Full Text
https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-023-00886-9
Systemic juvenile idiopathic arthritis (systemic JIA) is a severe disease with both systemic and joint inflammation. This study aims to identify predictors of disease evolution within the systemic JIA population enrolled in the Juvenile Inflammatory Rheumatism cohort (JIRcohort). […] Our results suggest that the earlier disease onset, the persistence of arthritis throughout the first year of disease evolution and the need of sDMARD might predict a persistent disease course. […] The patients of the persistent disease evolution group trended to have the disease onset at a younger age. […] Active arthritis was significantly more often seen in the persistent group at 12 months of diagnosis. […] The treatment frequency with bDMARD and sDMARD was higher in the persistent group. […] In our model parameters like young age at disease onset (6 years) and active arthritis at 12 months, were found to potentially have an influence on the risk of a persistent disease evolution during the first 24 months after diagnosis, even if the association was no longer statistically significant after adjusting for confounding factors. The need of sDMARD use may also predict a persistent disease course, reflecting a more severe phenotype.
#16 Disease evolution in systemic juvenile idiopathic arthritis: an international, observational cohort study through JIRcohort | Pediatric Rheumatology | Full Text
https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-023-00886-9
Systemic juvenile idiopathic arthritis (systemic JIA) is a severe disease with both systemic and joint inflammation. This study aims to identify predictors of disease evolution within the systemic JIA population enrolled in the Juvenile Inflammatory Rheumatism cohort (JIRcohort). […] Our results suggest that the earlier disease onset, the persistence of arthritis throughout the first year of disease evolution and the need of sDMARD might predict a persistent disease course. […] The patients of the persistent disease evolution group trended to have the disease onset at a younger age. […] Active arthritis was significantly more often seen in the persistent group at 12 months of diagnosis. […] The treatment frequency with bDMARD and sDMARD was higher in the persistent group. […] In our model parameters like young age at disease onset (6 years) and active arthritis at 12 months, were found to potentially have an influence on the risk of a persistent disease evolution during the first 24 months after diagnosis, even if the association was no longer statistically significant after adjusting for confounding factors. The need of sDMARD use may also predict a persistent disease course, reflecting a more severe phenotype.
#17 Predicting unfavorable long-term outcome in juvenile idiopathic arthritis: results from the Nordic cohort study | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-018-1571-6
The aim was to develop prediction rules that may guide early treatment decisions based on baseline clinical predictors of long-term unfavorable outcome in juvenile idiopathic arthritis (JIA). […] The feasibility of making long-term predictions of JIA outcome based on early clinical assessment is demonstrated. The prediction models have acceptable precision and require only readily available baseline variables. Further testing in other cohorts is warranted. […] The primary goal in the treatment of children with JIA is to achieve remission off medication, and the main implication of the current study is that prediction models may be useful in guiding decisions about treatment. […] In the Nordic JIA cohort, we have developed and evaluated prediction models for long-term unfavorable outcome with acceptable sensitivity and specificity based on variables easily available at baseline, which may guide individually tailored treatment. Prediction of long-term unfavorable outcome early in the disease course may be useful in deciding when to start aggressive treatment in JIA.
#18 Predicting unfavorable long-term outcome in juvenile idiopathic arthritis: results from the Nordic cohort study | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-018-1571-6
We have developed statistical models for predicting non-achievement of remission off medication, functional disability, and joint damage in children with JIA. The models are easy to use, and may provide a valuable tool to aid early treatment decisions on the need for DMARDs including biologic agents if validation in other JIA cohorts and across ethnicities can confirm our results.
#19 Predicting unfavorable long-term outcome in juvenile idiopathic arthritis: results from the Nordic cohort study | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-018-1571-6
We have developed statistical models for predicting non-achievement of remission off medication, functional disability, and joint damage in children with JIA. The models are easy to use, and may provide a valuable tool to aid early treatment decisions on the need for DMARDs including biologic agents if validation in other JIA cohorts and across ethnicities can confirm our results.
#20 FRI0559âVALIDATION OF NORDIC JUVENILE IDIOPATHIC ARTHRITIS CLINICAL PREDICTION MODELS IN A CANADIAN COHORT | Annals of the Rheumatic Diseases
https://ard.bmj.com/content/78/Suppl_2/975
Validation of clinical prediction tools for juvenile idiopathic arthritis (JIA) in populations different than those in which they were first developed is essential to understand their applicability across healthcare settings. […] To determine if clinical prediction tools to predict 1) non-achievement of remission off medication and 2) functional disability, developed in the Nordic cohort can be directly applied to JIA patients in the Canadian Research in Arthritis in Canadian Children emphasizing Outcomes (ReACCh-Out) cohort; and to assess performance of the prediction tools if model parameters are fine-tuned to the Canadian cohort. […] After fine-tuning of coefficients, the Nordic model for prediction of non-achievement of remission had similar prediction ability in Canadian patients with JIA. We could not confirm the validity of the Nordic model for prediction of functional disability in Canadian patients.
#21 FRI0559âVALIDATION OF NORDIC JUVENILE IDIOPATHIC ARTHRITIS CLINICAL PREDICTION MODELS IN A CANADIAN COHORT | Annals of the Rheumatic Diseases
https://ard.bmj.com/content/78/Suppl_2/975
Validation of clinical prediction tools for juvenile idiopathic arthritis (JIA) in populations different than those in which they were first developed is essential to understand their applicability across healthcare settings. […] To determine if clinical prediction tools to predict 1) non-achievement of remission off medication and 2) functional disability, developed in the Nordic cohort can be directly applied to JIA patients in the Canadian Research in Arthritis in Canadian Children emphasizing Outcomes (ReACCh-Out) cohort; and to assess performance of the prediction tools if model parameters are fine-tuned to the Canadian cohort. […] After fine-tuning of coefficients, the Nordic model for prediction of non-achievement of remission had similar prediction ability in Canadian patients with JIA. We could not confirm the validity of the Nordic model for prediction of functional disability in Canadian patients.
#22 Can We Predict Achievement of Clinically Inactive Disease and Sustained Remission in Children with Juvenile Idiopathic Arthritis? – ACR Meeting Abstracts
https://acrabstracts.org/abstract/can-we-predict-achievement-of-clinically-inactive-disease-and-sustained-remission-in-children-with-juvenile-idiopathic-arthritis/
Can We Predict Achievement of Clinically Inactive Disease and Sustained Remission in Children with Juvenile Idiopathic Arthritis? […] Identifying predictors for clinically inactive disease (CID) and sustained remission would allow rapid escalation of therapies for children less likely to achieve these states. This analysis assessed predictors of achievement of CID and sustained remission states over the first five years following diagnosis in children with JIA. […] Of 832 children, 70% were female and the majority had oligoarticular JIA (68%). At one year, 31% had achieved CID according to Wallaces preliminary criteria and 44% according to the cJADAS10 (26% CID on both). Within five years, 60% had ever achieved sustained remission on Wallaces preliminary criteria and 66% on the cJADAS10 (52% sustained remission on both).
#23 Can We Predict Achievement of Clinically Inactive Disease and Sustained Remission in Children with Juvenile Idiopathic Arthritis? – ACR Meeting Abstracts
https://acrabstracts.org/abstract/can-we-predict-achievement-of-clinically-inactive-disease-and-sustained-remission-in-children-with-juvenile-idiopathic-arthritis/
Can We Predict Achievement of Clinically Inactive Disease and Sustained Remission in Children with Juvenile Idiopathic Arthritis? […] Identifying predictors for clinically inactive disease (CID) and sustained remission would allow rapid escalation of therapies for children less likely to achieve these states. This analysis assessed predictors of achievement of CID and sustained remission states over the first five years following diagnosis in children with JIA. […] Of 832 children, 70% were female and the majority had oligoarticular JIA (68%). At one year, 31% had achieved CID according to Wallaces preliminary criteria and 44% according to the cJADAS10 (26% CID on both). Within five years, 60% had ever achieved sustained remission on Wallaces preliminary criteria and 66% on the cJADAS10 (52% sustained remission on both).
#24 Can We Predict Achievement of Clinically Inactive Disease and Sustained Remission in Children with Juvenile Idiopathic Arthritis? – ACR Meeting Abstracts
https://acrabstracts.org/abstract/can-we-predict-achievement-of-clinically-inactive-disease-and-sustained-remission-in-children-with-juvenile-idiopathic-arthritis/
There were different predictors for CID on the cJADAS10 vs. Wallaces preliminary criteria although similar predictors for sustained remission using either definition were identified. Children with poor functional ability, polyarticular JIA and younger age at initial presentation could be targeted with more aggressive treatment strategies than currently in practice to better control their disease.
#25 Can We Predict Achievement of Clinically Inactive Disease and Sustained Remission in Children with Juvenile Idiopathic Arthritis? – ACR Meeting Abstracts
https://acrabstracts.org/abstract/can-we-predict-achievement-of-clinically-inactive-disease-and-sustained-remission-in-children-with-juvenile-idiopathic-arthritis/
There were different predictors for CID on the cJADAS10 vs. Wallaces preliminary criteria although similar predictors for sustained remission using either definition were identified. Children with poor functional ability, polyarticular JIA and younger age at initial presentation could be targeted with more aggressive treatment strategies than currently in practice to better control their disease.
#26 FOREUM â Development of new tools for prediction and prevention of RA (PREDICT RA)
https://www.foreum.org/P017_pre_predictRA.cfm?projectid=153
Juvenile idiopathic arthritis (JIA) is a chronic inflammatory disease that can cause severe disability and even mortality with joint swelling, sensitivity, loss of motion and synovial tissue damage. […] We aim to define the role of T cell co-inhibitory receptors (co-IRs) for predicting the outcome of JIA and try to find a novel therapeutic target molecule. […] This is the first study showing the role of co-inhibitory receptors (checkpoint proteins) in the pathogenesis of JIA. […] Polyarticular JIA patients had a different coIR profile, having more CTLA-4, PD-1 and 4-1BB in their plasma than the other subtypes of JIA. […] We have shown that LAG-3 is an important molecule in the pathogenesis of oligoarticular JIA. […] We have shown that LAG3 agonists might be a novel therapeutic option for oligoarticular JIA patients in the future. […] This is the first study analysing these proteins in childhood arthritis and is hoped to lead to more work in the relevant area.
#27 FOREUM â Development of new tools for prediction and prevention of RA (PREDICT RA)
https://www.foreum.org/P017_pre_predictRA.cfm?projectid=153
Juvenile idiopathic arthritis (JIA) is a chronic inflammatory disease that can cause severe disability and even mortality with joint swelling, sensitivity, loss of motion and synovial tissue damage. […] We aim to define the role of T cell co-inhibitory receptors (co-IRs) for predicting the outcome of JIA and try to find a novel therapeutic target molecule. […] This is the first study showing the role of co-inhibitory receptors (checkpoint proteins) in the pathogenesis of JIA. […] Polyarticular JIA patients had a different coIR profile, having more CTLA-4, PD-1 and 4-1BB in their plasma than the other subtypes of JIA. […] We have shown that LAG-3 is an important molecule in the pathogenesis of oligoarticular JIA. […] We have shown that LAG3 agonists might be a novel therapeutic option for oligoarticular JIA patients in the future. […] This is the first study analysing these proteins in childhood arthritis and is hoped to lead to more work in the relevant area.
#28 Juvenile Idiopathic Arthritis (JIA) Symptoms, Causes & Treatment
https://my.clevelandclinic.org/health/diseases/10370-juvenile-idiopathic-arthritis
JIA affects each child differently. For some, it affects one or two joints, and the disease is easy to manage. For others, JIA may involve many joints and cause more severe or longer-lasting symptoms. […] With early detection and treatment, its possible to manage the arthritis, prevent joint damage, and allow normal or near-normal function for most children with JIA. […] JIA can last for months or years. Some children go into full remission, while others still have active symptoms into adulthood. Providers call this aging out of JIA and into adult arthritis. Theres really no way to predict whether this will happen. But your healthcare provider can help manage your childs condition, find effective treatments and set them up for success in the future.
#29 Juvenile Idiopathic Arthritis (JIA) Symptoms, Causes & Treatment
https://my.clevelandclinic.org/health/diseases/10370-juvenile-idiopathic-arthritis
JIA affects each child differently. For some, it affects one or two joints, and the disease is easy to manage. For others, JIA may involve many joints and cause more severe or longer-lasting symptoms. […] With early detection and treatment, its possible to manage the arthritis, prevent joint damage, and allow normal or near-normal function for most children with JIA. […] JIA can last for months or years. Some children go into full remission, while others still have active symptoms into adulthood. Providers call this aging out of JIA and into adult arthritis. Theres really no way to predict whether this will happen. But your healthcare provider can help manage your childs condition, find effective treatments and set them up for success in the future.
#30 Juvenile Idiopathic Arthritis (JIA) Symptoms, Causes & Treatment
https://my.clevelandclinic.org/health/diseases/10370-juvenile-idiopathic-arthritis
JIA affects each child differently. For some, it affects one or two joints, and the disease is easy to manage. For others, JIA may involve many joints and cause more severe or longer-lasting symptoms. […] With early detection and treatment, its possible to manage the arthritis, prevent joint damage, and allow normal or near-normal function for most children with JIA. […] JIA can last for months or years. Some children go into full remission, while others still have active symptoms into adulthood. Providers call this aging out of JIA and into adult arthritis. Theres really no way to predict whether this will happen. But your healthcare provider can help manage your childs condition, find effective treatments and set them up for success in the future.
#31 Early predictors of outcome in juvenile idiopathic arthritis – PubMed
https://pubmed.ncbi.nlm.nih.gov/14969057/
The definition and management of „early arthritis” in children differ from those in adults because juvenile idiopathic arthritis (JIA) is markedly different from adult rheumatoid arthritis. Since a significant proportion of patients with JIA develop articular damage and enter adult life with persistently active disease, it is important to predict early in the disease course the long-term outcome in order to tailor treatment to the risk of disability. […] In summary, greater severity/extension of arthritis at onset, symmetric disease, precocious hip/wrist involvement, the presence of rheumatoid factor, and prolonged active disease were the best predictors of a poor outcome. Specific correlates for systemic JIA were persistent systemic features and thrombocytosis at 6 months following presentation, whereas joint symmetry and a higher erythrocyte sedimentation rate at onset were associated with a more severe course in oligoarticular JIA. However, although data is accumulating on prognostic factors in JIA, prediction of long-term outcome in the first few months remains difficult. To better define prognostic factors in future analyses, a considerable effort should be made to increase standardization among studies. Furthermore, a radiographic scoring system and a set of remission criteria specific for JIA should be developed.
#32 Early predictors of outcome in juvenile idiopathic arthritis – PubMed
https://pubmed.ncbi.nlm.nih.gov/14969057/
The definition and management of „early arthritis” in children differ from those in adults because juvenile idiopathic arthritis (JIA) is markedly different from adult rheumatoid arthritis. Since a significant proportion of patients with JIA develop articular damage and enter adult life with persistently active disease, it is important to predict early in the disease course the long-term outcome in order to tailor treatment to the risk of disability. […] In summary, greater severity/extension of arthritis at onset, symmetric disease, precocious hip/wrist involvement, the presence of rheumatoid factor, and prolonged active disease were the best predictors of a poor outcome. Specific correlates for systemic JIA were persistent systemic features and thrombocytosis at 6 months following presentation, whereas joint symmetry and a higher erythrocyte sedimentation rate at onset were associated with a more severe course in oligoarticular JIA. However, although data is accumulating on prognostic factors in JIA, prediction of long-term outcome in the first few months remains difficult. To better define prognostic factors in future analyses, a considerable effort should be made to increase standardization among studies. Furthermore, a radiographic scoring system and a set of remission criteria specific for JIA should be developed.
#33 Long-term outcomes and disease course of children with juvenile idiopathic arthritis in the ReACCh-Out cohort: a two-centre experience
https://pmc.ncbi.nlm.nih.gov/articles/PMC7733713/
To assess long-term outcomes of children with JIA diagnosed in the biologic era. […] At the last follow-up visit at a median age of 16.9 years, 47% were in remission off medications, 25% in remission on medications and 27% had active disease; 51% were on at least one anti-rheumatic medication (22% on biologics). […] Relative to historical cohorts, this study suggests a reduction in JIA permanent damage, a more favourable prognosis for systemic JIA and a lower progression to oligoarthritis extended category. However, in an era of biologic therapy, one in four patients with JIA still enter adulthood with active disease and one in two still on treatment. […] Relative to historical cohorts, young adults who were diagnosed with JIA early in the biologic era at two Canadian centres in this study had higher rates of remission and lower rates of permanent joint damage (1% required joint replacements).
#34 Long-term outcomes and disease course of children with juvenile idiopathic arthritis in the ReACCh-Out cohort: a two-centre experience
https://pmc.ncbi.nlm.nih.gov/articles/PMC7733713/
Our study found patients with systemic JIA had a good long-term prognosis with 70% in remission off medications and only 9% with active disease at a median of 5.7 years after diagnosis. […] This long-term follow-up of an inception cohort diagnosed early in the biologic era suggests a reduction in permanent damage across JIA categories, a more favourable prognosis for systemic JIA and a lower progression to oligoarthritis extended category than in historical cohorts. However, one in four patients still had disease activity in early adulthood and one in two were receiving anti-rheumatic medications.
#35 Long-term outcomes and disease course of children with juvenile idiopathic arthritis in the ReACCh-Out cohort: a two-centre experience
https://pmc.ncbi.nlm.nih.gov/articles/PMC7733713/
To assess long-term outcomes of children with JIA diagnosed in the biologic era. […] At the last follow-up visit at a median age of 16.9 years, 47% were in remission off medications, 25% in remission on medications and 27% had active disease; 51% were on at least one anti-rheumatic medication (22% on biologics). […] Relative to historical cohorts, this study suggests a reduction in JIA permanent damage, a more favourable prognosis for systemic JIA and a lower progression to oligoarthritis extended category. However, in an era of biologic therapy, one in four patients with JIA still enter adulthood with active disease and one in two still on treatment. […] Relative to historical cohorts, young adults who were diagnosed with JIA early in the biologic era at two Canadian centres in this study had higher rates of remission and lower rates of permanent joint damage (1% required joint replacements).
#36 Predicting unfavorable long-term outcome in juvenile idiopathic arthritis: results from the Nordic cohort study | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-018-1571-6
The aim was to develop prediction rules that may guide early treatment decisions based on baseline clinical predictors of long-term unfavorable outcome in juvenile idiopathic arthritis (JIA). […] The feasibility of making long-term predictions of JIA outcome based on early clinical assessment is demonstrated. The prediction models have acceptable precision and require only readily available baseline variables. Further testing in other cohorts is warranted. […] The primary goal in the treatment of children with JIA is to achieve remission off medication, and the main implication of the current study is that prediction models may be useful in guiding decisions about treatment. […] In the Nordic JIA cohort, we have developed and evaluated prediction models for long-term unfavorable outcome with acceptable sensitivity and specificity based on variables easily available at baseline, which may guide individually tailored treatment. Prediction of long-term unfavorable outcome early in the disease course may be useful in deciding when to start aggressive treatment in JIA.
#37 Predicting unfavorable long-term outcome in juvenile idiopathic arthritis: results from the Nordic cohort study | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-018-1571-6
The aim was to develop prediction rules that may guide early treatment decisions based on baseline clinical predictors of long-term unfavorable outcome in juvenile idiopathic arthritis (JIA). […] The feasibility of making long-term predictions of JIA outcome based on early clinical assessment is demonstrated. The prediction models have acceptable precision and require only readily available baseline variables. Further testing in other cohorts is warranted. […] The primary goal in the treatment of children with JIA is to achieve remission off medication, and the main implication of the current study is that prediction models may be useful in guiding decisions about treatment. […] In the Nordic JIA cohort, we have developed and evaluated prediction models for long-term unfavorable outcome with acceptable sensitivity and specificity based on variables easily available at baseline, which may guide individually tailored treatment. Prediction of long-term unfavorable outcome early in the disease course may be useful in deciding when to start aggressive treatment in JIA.
#38 Can We Predict Achievement of Clinically Inactive Disease and Sustained Remission in Children with Juvenile Idiopathic Arthritis? – ACR Meeting Abstracts
https://acrabstracts.org/abstract/can-we-predict-achievement-of-clinically-inactive-disease-and-sustained-remission-in-children-with-juvenile-idiopathic-arthritis/
There were different predictors for CID on the cJADAS10 vs. Wallaces preliminary criteria although similar predictors for sustained remission using either definition were identified. Children with poor functional ability, polyarticular JIA and younger age at initial presentation could be targeted with more aggressive treatment strategies than currently in practice to better control their disease.